Case Reports Korean Circulation J 2000;307:876-880 관상동맥조영술후폐동맥혈전증으로발현된 S 단백단독결핍증 1 례 유병현 김용주 박원석 김명숙 박현옥 진승원전두수 김종진 박준철 김재형 홍순조 최규보 A Case of Isolated Protein S Deficiency, Complicated by Acute Pulmonary Thromboembolism after Coronary Angiography Byung Hyun Yoo, MD, Yong Joo Kim, MD, Won Seok Park, MD, Myung Sook Kim, MD, Hyun Ok Park, MD, Seung Won Jin, MD, Doo Soo Chon, MD, Jong Jin Kim, MD, Jun Cheol Park, MD, Jae Hyung Kim, MD, Soon Jo Hong, MD and Kyu Bo Choi, MD Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea ABSTRACT Protein S is a cofactor for activated protein C in degradation of factor Va and a. Deficiency of protein S increases the risk of venous thrombosis and pulmonary thromboembolism. We report a case with isolated protein S deficiency who developed acute pulmonary thromboembolism after a routine coronary angiography. A 35-year-old woman was referred for evaluation of exertional chest pain. Coronary angiography was done by right femoral artery approach. After removal of compressive dressing, she suddenly felt dyspnea. Pulmonary thromboembolism was diagnosed by immediate transthoracic echocardiography and dynamic chest CT. Thrombolysis with urokinase and heparinization was done successfully. After 6-month-warfarinization, test for evaluation for hypercaogulable state was done. Total protein S antigen, free protein S antigen and protein S activity were all decreased. Protein C activity and mass, antithrombin, anticardiolipin test, lupus anticoagulant antibody, antiphospholipid antibody, factor Leiden mutation test, fibrinogen, FDP, and homocyteine levels were all normal. Korean Circulation J 2000;307:876-880 KEY WORDSIsolated protein S deficiency Pulmonary thromboembolism. 서 론 876
증례 A B Fig. 1. AThe initial echocardiography showed normal findings. BEchocardiography after development of acute pulmonary thromboembolism showed marked dilatation of right ventricle RV & flattening of interventricular septum. LVleft ventricle. 877
A B Fig. 2. A Dynamic chest CT showed large filling defects (arrow) in both main pulmonary arteries. B Follow-up chest CT showed marked regression of filling defects (arrow head) compared to previous study. 53.3 mmhg, pco2 26.6 mmhg, HCO3 17.3 meq/l, 이었다. SaO2 90.7% 이었다. 산소 7L 공급 후 시행한 동맥혈 6개월간 warfarin을 투여하였고 warfarin 중지 한달 가스 분석상 ph 7.44, po2 68.2 mmhg, pco2 31.0 후에 측정한 C 단백 활성도 112%, C 단백 항원 116% mmhg, HCO3 20.8 meq/l, SaO2 94.7% 이었다. 심 (70 140), S 단백 활성도 28%, 전체 S 단백 항원 전도상 동성 빈맥이 관찰되었다. 흉부 X-선 촬영상 특 41%(70 140), 유리형 S 단백 항원 65%(70 130), 이 소견 없었으며, 심초음파상 우심실의 확장 및 벽운 antithrombin Ⅲ 29.2 mg/dl(19 31), 출혈시간 2분 동 감소, 심실중격의 편평화가 관찰되어 폐동맥 고혈압 (1 4분), 응고시간 5분(1 5분), homocysteine 6.8 이 의심되었다(Fig. 1-B). 흉부단층촬영(Fig. 2-A) μmol/l(4.45 12.42), lupus anticoagulant antibody 상 양쪽 폐동맥의 충만 결손(filling defect)이 관찰되 0.91 LAC Screen(0.8 1.2), anticardiolipin antibody 어 폐동맥 혈전증이 강력히 의심되었다. 유로키나제 3 (IgG/M)(-/-), antiphospholipid antibody(igg)(-), 백만 단위를 정주 하였고(150만 단위를 일시 투여한 fibrinogen 370 mg/dl(200 400), factor V Leiden 후 150만 단위를 1시간 동안 투여함) 동시에 헤파린 mutation test negative, FDP 10 μg/dl(<10), fac- 을 정주하였다. 유로키나제 투여 10시간 후의 혈압은 tor Ⅷ 105%(60 140)이었다(Table 1). 그외에 추 100/70 mmhg, 맥박수는 분당 108회, 호흡수는 분당 적 흉부단층촬영이나 폐관류검사는 환자가 거부하여 20회로 빈호흡이 호전되었다. 유로키나제 투여 5일후 시행하지 못하였고, 또 가족이나 친척들에 대한 과응고 시행한 동맥혈가스분석상 ph 7.46, pco2 38.3 mmhg, 에 관한 검사도 거부하여 시행하지 못하였다. 환자는 po2 76.1 mmhg, HCO3 26.9 meq/l, SaO2 96.0%이 현재 특별한 약제 투여는 하지 않고 있으며 외래 추적 었고, 혈압은 110/80 mmhg, 맥박수는 분당 85회, 호 관찰중이다. 흡수는 분당 20회로 안정화되었다. 관상동맥조영술 후 고 3일째 실시한 폐관류검사(Fig. 3)상 양측 폐의 충만결 안 손이 보였다. 관상동맥조영술 후 13병일 째 실시한 양 하지정맥조영술상 정상이었다. 관상동맥조영술 후 18 S 단백은 C 단백의 보조인자로서 활성화된 C 단백 병일 째에 시행한 추적 흉부단층촬영(Fig. 2-B)상 충 과 복합체를 이루어, factor Va, factor Ⅷa를 분해시 만결손의 현저한 감소를 보였다. 유로키나제와 헤파린 켜 항응고 작용을 하게 한다. S 단백의 구성은 C4b- 을 사용하기 전에 혈액 채취를 실시했으며, 검사상 binding protein과 60%가 결합하고, 나머지 40%가 VDRL(-), antiphospholipid Ab(-), C 단백 활성도 유리형으로 존재하며, 유리형이 기능적으로 활성된 형 75.0 %(73 142), S 단백 활성도 41.0%(60 140) 태이다.1) 878 Korean Circulation J 2000;30(7):876-880
Fig. 3. Lung perfusion scan showed multifocal irregular and triangular perfusion defects, suggesting multiple pulmonary embolism. Table 1. Laboratory findings Refernce value VDRL Antiphospholipid Ab Protein C 75.0% 73142 Protein S 41.0% 60140 6 wafarin Protein C 112% 73142 Protein C antigen 116% 70140 Protein S 28% 60140 Protein S antigen total 41% 70140 Protein S antigen free 65% 70130 Antithrombin 29.2 mg/dl 1931 Fibrinogen 370 mg/dl 200400 FDP 10 g/dl 10 2 14 5 15 Homocysteine 6.8 mol/l 4.4512.42 Lupus anticoagulant 0.91 LAC Screen antibody 0.81.2 Anticardiolipin antibody IgG/M / / Antiphospholipid antibody IgG Factor Leiden mutation test negative negative Factor 105% 60140 879
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